What causes CFRD

[lflatford]

My daughter may be enzyme deficient even after taking her enzymes regularly. We have a test scheduled next week to check it out. My husband was told by someone that this can lead to CFRD. Is this true? If so any other info would help. If not what are the causes of CFRD?

 

[miesl]

<i>The pancreas shows fatty replacement, fibrosis and a reduction in the number and function of secretory cells. These include the ß-cells within the islets of Langerhans which produce insulin. These changes are likely to result from obstruction of pancreatic ducts by abnormal, thick secretions. Autopsy studies, however, have not shown that fibrotic islet changes are significantly greater in patients with CFRD than in patients with CF without diabetes. Such studies suggest that CFRD is more than merely severe fibrosis induced islet damage. Islet amyloid polypeptide is co-secreted with insulin in normal health but does not accumulate within the islet. Amyloid deposition is not seen in type 1 diabetes or in chronic pancreatitis but is seen in type 2 diabetes. Autopsy studies did not find islet amyloid in any of the patients with cystic fibrosis without CFRD but this deposition was found in 17% of the borderline diabetic cases and in 69% of the cases with full CFRD. There is thus a suggested similarity between CFRD and type 2 diabetes. This has led to the speculation that impaired glucose tolerance is very common in adults with CF because of fibrotic damage to the islet, but may only progress to full CFRD in patients with a genetic defect in insulin secretion similar to that found in type 2 diabetes (Moran et al, 1998).</i>

From <a target=new class=ftalternatingbarlinklarge href="http://www.cysticfibrosismedicine.com/htmldocs/CFText/diabetes.htm">this article</a>.

Basically - they aren't quite sure. It does however have a greater rate of occurance in pancreatic insufficient people (and is more common with double DF508).

 

[lflatford]

Thank you Miesl. The info is a little confusing yet helpful. My daughter is a double F508, so it may turn out she has this issue. Will just have to wait for the test results.